Esthesioneuroblastoma. Long term outcome and patterns of failure—the University of Virginia experience

Abstract

Background. Esthesioneuroblastoma is a rare tumor arising from olfactory epithelium. This retrospective review analyzed the patterns of failure and long term outcome of patients with esthesioneuroblastoma evaluated at a single institution. Methods. Forty patients with esthesioneuroblastoma were evaluated at the University of Virginia, with a median follow‐up of 130 months. In most cases, treatment consisted of combined‐modality therapy, including radiotherapy and surgery for Stages A and B disease and the addition of chemotherapy for Stage C disease. Fifteen patients received chemotherapy that included cyclophos‐phamide plus vincristine. Thirty‐eight patients received radiotherapy, with a median dose of 50 Gy. Initial surgery for 23 patients included craniofacial resection, whereas the remainder had less extensive surgery (3 had no initial surgery). Five patients were salvaged with high dose chemotherapy and autologous bone marrow transplantation (CTX/BMT). Results. Actuarial survivals at 5, 10, and 15 years are 78%, 71%, and 65% respectively. Fifty‐five percent of patients failed therapy, and 68% of the failures were locore‐gional. Thirty‐nine percent of recurrences occurred later than 5 years from diagnosis. Three of the five patients were successfully salvaged with CTX/BMT compared with four of seventeen patients who underwent conventional salvage therapy. Conclusions. Esthesioneuroblastoma is associated with long term survival and late recurrences. Multimodality therapy should be used initially. Durable remissions of failures can be achieved with CTX/BMT.

Esthesioneuroblastoma: a meta-analysis and review

“…In the studies with survival data according to the N stage, 2,8,60,61,64,69,78 only 29% of nodepositive patients were treated successfully, compared with 64% of N0 patients, a significant difference (odds ratio 5.1 [95% CI 1.6-17.0]).…”

“…The 26 studies 2,[4][5][6]8,43,[54][55][56][57][58][60][61][62][63][64][65][66][68][69][70][71][72][73]77,78 reported on 390 patients with ENB. Overall survival at 5 years could be extracted from 25 studies; the mean was 45% (SD 22), with extremes of 86% 58 and zero.…”

“…2,6,[54][55][56][57][58][61][62][63][64][65][66][68][69][70][71][72][73]77,78 The distribution was 12% stage A, 27% stage B, and 61% stage C. Mean survival for these three groups was 72% (SD 41), 59% (SD 44), and 47% (SD 16), respectively. Four studies 2,4-6 used the Dulguerov and Calcaterra staging with the following distribution: 25% T1, 25% T2, 33% T3, and 17% T4.…”

“…2,4,61,63 The theoretical advantage that preoperative radiotherapy will reduce an unresectable tumour to a resectable size has never been clearly demonstrated. 64 Standard radiotherapeutic techniques include external megavoltage beam and a three-field technique; an anterior port is combined with wedged lateral fields to provide a homogeneous dose distribution. The dose ranges from 5500 cGy to 6500 cGy and in most cases is above 6000 cGy.…”

Neuroectodermal Neoplasms of the Head and Neck with Emphasis on Neuroendocrine Carcinomas

“…Despite the extensive nature of this procedure, other than anosmia, the resultant functional and cosmetic deficits are minimal. Complete surgical resection, often followed by supplemental radiation or chemotherapy, results in a 5-year cure rate of approximately 75% (94). Although counterintuitive for a tumor with blastoma in its name, 5-year disease-free survivals should not be equated with cure.…”